THE PATHOLOGIC SPECTRUM OF HUNTINGTONʼS CHOREA

Abstract

The pathologic findings in 21 cases of Huntington''s chorea have been described. Striatal degeneration was invariably the dominant lesion. Histologically, the lesion was characterized by diffuse loss of small ganglion cells and marked reactive astrocytosis. The large ganglion cells of the striatum were substantially spared by the process. Although shrinkage and gliosis were sometimes rather more obvious in the caudate nucleus than in the putamen, both structures were, for practical purposes, similarly involved. Each division showed a virtually uniform involvement of the entire substance, aside from a common tendency for the most inferior parts of the anterior segment of each to be appreciably less affected. The globus pallidus frequently showed a mild or moderate secondary atrophy, with no evidence of loss of nerve cells. Less often, similar changes were noted in the centromedian nucleus of the thalamus. Although pronounced degrees of gyral atrophy often occurred, wasting was slight in seven cases. The microscopic changes in the cerebral cortex varied correspondingly, but were always mild as compared with the striatal lesion. The cortical degeneration closely resembles that of simple senile atrophy. The dentate nucleus of the cerebellum showed an obvious loss of nerve cells in 9 cases, and in three this was conspicuous. In 2 cases there were marked atrophy of the nuclei pontis. Other parts of the nervous system, including the subthalamic area, showed no significant changes. The general pathologic findings revealed no evidence of any underlying systemic disturbance.